Objective: Bringing into question a case of left ventricular non-compaction dilated cardiomyopathy, presented to the emergency room with dyspnea and fatigue.
Case presentation: The case of a 32-year old admitted to our clinic for heart failure phenomena is presented. A transthoracic echocardiography was performed by the emergency service, where suspicion of DCM by non-compaction of LV was raised. The patient was admitted for investigations, diagnosis and specialised treatment. The transthoracic echocardiography revealed specific modifications of LV non-compaction DCM. Segments involved are mid ventricular (especially inferior and lateral ones) and apical with evidence of direct blood flow from the ventricular cavity into deep intertrabecular recesses by Doppler color. Biological analyzes have revealed the following: CK-MB=2.12 ng/ml, TnI=0.018 ng/ml, D-dimers=2.55 ug/ ml, NTproBNP=4074 pg/ml; The presence of Atg anti HBs at high positive titer. Otherwise, normal values. Cardiothoracic radiography showed: excessive dilation of the right atrium and elongation of the left ventricle. Holter ECG: sinus rhythm throughout the monitoring period with frequent ventricular extrasystoles, with 2 episodes of NSVT (3 consecutive PVC) with predominantly high heart rate. ECG reveals sinus rhythm with frequent, monomorphic, isolated ventricular extrasystoles. Specialized treatment has been instituted for heart failure. Family members have been evaluated. Cardiac MRI could not be performed. The established diagnosis was left ventricular non-compaction DCM and standard treatment with betablocker, ACE, diuretic, anticoagulant, amiodarone was instituted. Since his initial diagnosis, the patient has returned to the emergency room multiple times with volume overload due to medication non-compliance. Each time after being optimized, he returned to his baseline functional status where he can perform his activities of daily living without significant difficulties.
Conclusions: Myocardial noncompaction is a rare primary cardiomyopathy with genetic transmission affecting the left ventricle or both ventricles. It is characterized by the persistence of the fetal spongiform structure with a deep trabecular myocardium with deep inter-trabecular recesses that communicates freely with the cavity of the left ventricle, but not with the coronary circulation. The pathogenic mechanism of this disease consists of stopping the cardiomyocyte compaction process during intrauterine life. Early diagnosis is desirable to take symptomatic and prophylactic therapeutic measures, possibly implanting intracardiac devices to prevent sudden death and discuss cardiac